Case Report
J Clin Ultrasound 20:612-614, NovemberiDecember 1992 CCC 0091-2751/92/090612-03 0 1992 by John Wiley & Sons, Inc.
Infective Endocarditis in Hypertrophic Obstructive Cardiomyopathy Ming-Ren Chen, MD
Infective endocarditis (IE) is a rare complication of hypertrophic obstructive cardiomyopathy (HOCM). In adults, the incidence is about 5%.l Up to now, there have been only 43 cases reand all of them were ported in the adults. This report describes a pediatric case of HOCM with IE and its cerebral complication. CASE REPORT
An 11-year, 4-month-old girl was admitted with the chief complaints of generalized malaise, body weight loss, and cold sweating at night for 1 month. Past history revealed a heart murmur since early childhood. Physical examination showed a chronicly ill, pale, febrile girl. A Grade 3 pansystolic murmur and a Grade 2 diastolic murmur at the apex were audible. Another Grade 2 systolic ejection murmur was noted at left and right middle sternal border. The liver was 2 cm palpable below right costal margin, but the spleen was not palpable. An electrocardiogram showed left superior axis deviation, atrial and ventricular premature beats, intraventricular conduction disturbance, and left ventricular wall ischemia. Moderate cardiomegaly was noted on the chest X-ray. Abdominal echogram showed moderate amount of ascites. M-mode and two-dimensional echocardiography showed a very thick interventricular septum (30 mm) disproportinate to the left ventricular posterior wall (9 mm) (Figure 11, systolic, anterior motion of the anterior mitral leaflet (large arrow in Figure l), and 3 vegetations on the atrial surface of anterio- posterior mitral leaflet and on the ventricular septa1 surface of the subaortic area, respectively (small arrows in Figure
From the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China. For reprints contact Ming-Ren Chen, MD, Department of Pediatrics, Mackay Memorial Hospital, 92, See 2, Chung San North Road, Taipei, Taiwan, Republic of China.
612
1). Early to midsystolic closure of the aortic cusps was also noted (arrow in Figure 2). Color Doppler mapping and continuous wave doppler echocardiography revealed a severe degree of mitral regurgitation and a moderate degree of subaortic stenosis (pressure gradient: 62 mm Hg). Infective endocarditis was proved by blood culture. Four blood samples from different sites all grew a-hemolytic strepcocci sanguis 11. Aqueous penicillin plus garamycin was chosen as the medical regimen. On the sixth admission day, the patient complained of headache and chills. Her neck was rigid and the fundi of her eyes showed hemorrhagic spots. Nevertheless, the vital signs were stable. Emergent brain computerized tomography showed a large area of intracranial hemorrhage in the right temporal and right parietal lobes with perifocal edema. An abscess was also seen in the right frontal lobe. In addition, right to left midline shift with subarachnoid hemorrhage involving the right sylvian fissure and right ambient cistern were seen. After supportive therapy was initiated, surgical treatment for the heart and the brain problems was recommended. Unfortunately, her family refused our recommendation, and she was taken from the hospital without further treatment.
DISCUSSION
Although the underlying disease spectrum of IE has changed in recent years, HOCM is still thought to be infrequently complicated by infective end0~arditis.l~ Because HOCM is a disorder of the myocardium and not the endocardium, the risk of IE is thought to be 10w.l~ In this patient,-fever, pallor, a chronicly ill appearance, and the heart murmur raised the suspicion of IE. The finding of vegetations in the echocardiographic studies and the positive cultures confirmed the diagnosis. Except for the pansystolic murmur of subaortic obstruction, the
INEFFECTIVE ENDOCARDITIS IN HOCM
613
FIGURE 1. M-mode and two-dimensional echocardiograms (long axis view) show a very thick interventricular septum (30 mm) disproportionate to the left ventricular posterior wall (9 mrn). The systolic and diastolic left ventricular internal dimensions are 27 mm and 47 mm. respectively. A large arrow indicates systolic anterior motion of the anterior mitral leaflet. Three vegetations are marked with small arrows. (LA: left atrium; LV: left ventricle.)
diastolic murmur at the apex may be due to relative mitral stenosis. With echocardiography, the M-mode technique helped in the diagnosis of the disproportionate ratio of interventricular septum and posterior left ventricular wall thickness, the systolic anterior motion of the anterior mitral leaflet, and the midsystolic closure of the aortic cusps. the sensitivity of two-dimensional echocardiography in the detection of vegetations in IE is superior to that of M-mode and ranges up to 83%.14 The adjunctive use of Doppler techniques to di-
agnose the mitral regurgitation and subaortic obstruction provides more details. Because HOCM is a disorder of the myocardium and not the endocardium, it has been considered to have low risk of IE. In fact, the Venturi effect is responsible for the vegetations on the atrial surface of mitral leaflets in cases of mitral regurgitation. The direct trauma resulting from septal-anterior mitral leaflet contact may predispose to infection and vegetation formation. Therefore, hemodynamically HOCM is similar to a valvular disease, and the risk of IE cannot be
FIGURE 2. M-mode and two-dimensional echocardiograms (long axis view) show early- to mid-systolic closure of aortic cusps (arrow). (LAand LV: same as in Figure 1; RV: right ventricle; AO: aorta.)
VOL. 20, NO. 9, NOVEMBERiDECEMBER 1992
CASE REPORT: CHEN
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neglected. In HOCM with IE, vegetations can be found on the anterior mitral valve leaflet and aortic cusps with equal frequency, and less commonly in the left ventricular outflow tract,1712 such as in our patient. Adequate intravenous antibiotic administration for at least 4 weeks and up to 6 weeks or longer is required to sterilize vegetations. In this patient, because of embolic and mycotic complications, surgical intervention for myomectomy and removal of vegetations to prevent recurrent episodes of IE and systemic embolic effect was indicated. This case is not only a rare pediatric HOCM with IE, but it also demonstrates the invaluable role of echocardiography in the diagnosis of HOCM and its complications, especially in the case of IE. REFERENCES 1. Mazzoli M, Tefani 0, Vergassola R: Infective endocarditis: A complication of idiopathic hypertrophic subaortic stenosis (author’s translation). G Ital Cardiol 10:1228- 1233, 1980. 2. Novikov IuI, Polubentseva EI, Bespalova GN: Infectious endocarditis in hypertrophic cardiomyopathy. Ter Arkh 62235-89, 1990. 3. Stulz P, Zimmerli W, Mihatsch J , et al: Recurrent infective endocarditis in idiopathic hypertrophic subaortic stenosis. Thorac Cardiouasc Surg 37: 99- 102, 1989. 4. Carpenter PM, Atai M, Hoit B: Aneurysm of the mitral valve in a patient with hypertrophic cardiomyopathy. A m J Cardiouasc Pathol 2:273-276, 1988. 5. Winkelmann M, Curtius JM, Hoffmann V, et al:
Infectious endocarditis in hypertrophic obstructive cardiomyopathy. Z Kardiol 75:505-508, 1986. 6. Khekimian A, Pavlov TS: Case of infectious endocarditis in hypertrophic cardiomyopathy. Vutr Boles 25:104-107, 1986. 7. Petitalot JP, Allal J , Bordage J P , et al: Diagnosis and echocardiographic course of infectious endocarditis in obstructive cardiomyopathy. Ann Cardiol Angeiol (Paris) 34:353-356, 1985. 8. Zimlichman R, Ovsyshcher IA: Infective endocarditis in hypertrophic cardiomyopathy: Case report and review of literature 1961-1982. Mt Sinai J Med 51:614-619, 1984. 9. Ovsyshcher IA, Zimlichman R: Infective endocarditis in hypertrophic cardiomyopathy secondary to amiodarone treatment [letter]. Chest 832333, 1983. 10. Martinez-Orozco F, Ancochea L, Valls V, et al: Subacute infectious endocarditis in a n elderly patient suffering from obstructive cardiomyopathy. Rev Clin Esp 166:245-247, 1982. 11. Moiseev VS, Zhdanova NS, Kosmachev NN, et al: Infectious endocarditis in hypertrophic (muscular) subaortic stenosis. Klin Med (Mosk) 60238-91, 1982. 12. Chagnac A, Rudniki C, Loebel H, et al: Infectious endocarditis in idiopathic hypertrophic subaortic stenosis: Report of three cases and review of the literature. Chest 81:346-349, 1982. 13. McKinsey DS, Ratts TE, Bisno AL: Underlying cardiac lesions in adults with infective endocarditis. The changing spectrum. Am J Med 82:681688, 1987. 14. Lutas EM, Roberts RB, Devereaux RB, Prieto LM: Relation between the presence of echocardiographic vegetations and the complication rate in infective endocarditis. Am Heart J 112:107-113, 1986.
JOURNAL
OF CLINICAL ULTRASOUND
J Clin Ultrasound 20:612-614, NovemberiDecember 1992 CCC 0091-2751/92/090612-03 0 1992 by John Wiley & Sons, Inc.
Infective Endocarditis in Hypertrophic Obstructive Cardiomyopathy Ming-Ren Chen, MD
Infective endocarditis (IE) is a rare complication of hypertrophic obstructive cardiomyopathy (HOCM). In adults, the incidence is about 5%.l Up to now, there have been only 43 cases reand all of them were ported in the adults. This report describes a pediatric case of HOCM with IE and its cerebral complication. CASE REPORT
An 11-year, 4-month-old girl was admitted with the chief complaints of generalized malaise, body weight loss, and cold sweating at night for 1 month. Past history revealed a heart murmur since early childhood. Physical examination showed a chronicly ill, pale, febrile girl. A Grade 3 pansystolic murmur and a Grade 2 diastolic murmur at the apex were audible. Another Grade 2 systolic ejection murmur was noted at left and right middle sternal border. The liver was 2 cm palpable below right costal margin, but the spleen was not palpable. An electrocardiogram showed left superior axis deviation, atrial and ventricular premature beats, intraventricular conduction disturbance, and left ventricular wall ischemia. Moderate cardiomegaly was noted on the chest X-ray. Abdominal echogram showed moderate amount of ascites. M-mode and two-dimensional echocardiography showed a very thick interventricular septum (30 mm) disproportinate to the left ventricular posterior wall (9 mm) (Figure 11, systolic, anterior motion of the anterior mitral leaflet (large arrow in Figure l), and 3 vegetations on the atrial surface of anterio- posterior mitral leaflet and on the ventricular septa1 surface of the subaortic area, respectively (small arrows in Figure
From the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China. For reprints contact Ming-Ren Chen, MD, Department of Pediatrics, Mackay Memorial Hospital, 92, See 2, Chung San North Road, Taipei, Taiwan, Republic of China.
612
1). Early to midsystolic closure of the aortic cusps was also noted (arrow in Figure 2). Color Doppler mapping and continuous wave doppler echocardiography revealed a severe degree of mitral regurgitation and a moderate degree of subaortic stenosis (pressure gradient: 62 mm Hg). Infective endocarditis was proved by blood culture. Four blood samples from different sites all grew a-hemolytic strepcocci sanguis 11. Aqueous penicillin plus garamycin was chosen as the medical regimen. On the sixth admission day, the patient complained of headache and chills. Her neck was rigid and the fundi of her eyes showed hemorrhagic spots. Nevertheless, the vital signs were stable. Emergent brain computerized tomography showed a large area of intracranial hemorrhage in the right temporal and right parietal lobes with perifocal edema. An abscess was also seen in the right frontal lobe. In addition, right to left midline shift with subarachnoid hemorrhage involving the right sylvian fissure and right ambient cistern were seen. After supportive therapy was initiated, surgical treatment for the heart and the brain problems was recommended. Unfortunately, her family refused our recommendation, and she was taken from the hospital without further treatment.
DISCUSSION
Although the underlying disease spectrum of IE has changed in recent years, HOCM is still thought to be infrequently complicated by infective end0~arditis.l~ Because HOCM is a disorder of the myocardium and not the endocardium, the risk of IE is thought to be 10w.l~ In this patient,-fever, pallor, a chronicly ill appearance, and the heart murmur raised the suspicion of IE. The finding of vegetations in the echocardiographic studies and the positive cultures confirmed the diagnosis. Except for the pansystolic murmur of subaortic obstruction, the
INEFFECTIVE ENDOCARDITIS IN HOCM
613
FIGURE 1. M-mode and two-dimensional echocardiograms (long axis view) show a very thick interventricular septum (30 mm) disproportionate to the left ventricular posterior wall (9 mrn). The systolic and diastolic left ventricular internal dimensions are 27 mm and 47 mm. respectively. A large arrow indicates systolic anterior motion of the anterior mitral leaflet. Three vegetations are marked with small arrows. (LA: left atrium; LV: left ventricle.)
diastolic murmur at the apex may be due to relative mitral stenosis. With echocardiography, the M-mode technique helped in the diagnosis of the disproportionate ratio of interventricular septum and posterior left ventricular wall thickness, the systolic anterior motion of the anterior mitral leaflet, and the midsystolic closure of the aortic cusps. the sensitivity of two-dimensional echocardiography in the detection of vegetations in IE is superior to that of M-mode and ranges up to 83%.14 The adjunctive use of Doppler techniques to di-
agnose the mitral regurgitation and subaortic obstruction provides more details. Because HOCM is a disorder of the myocardium and not the endocardium, it has been considered to have low risk of IE. In fact, the Venturi effect is responsible for the vegetations on the atrial surface of mitral leaflets in cases of mitral regurgitation. The direct trauma resulting from septal-anterior mitral leaflet contact may predispose to infection and vegetation formation. Therefore, hemodynamically HOCM is similar to a valvular disease, and the risk of IE cannot be
FIGURE 2. M-mode and two-dimensional echocardiograms (long axis view) show early- to mid-systolic closure of aortic cusps (arrow). (LAand LV: same as in Figure 1; RV: right ventricle; AO: aorta.)
VOL. 20, NO. 9, NOVEMBERiDECEMBER 1992
CASE REPORT: CHEN
614
neglected. In HOCM with IE, vegetations can be found on the anterior mitral valve leaflet and aortic cusps with equal frequency, and less commonly in the left ventricular outflow tract,1712 such as in our patient. Adequate intravenous antibiotic administration for at least 4 weeks and up to 6 weeks or longer is required to sterilize vegetations. In this patient, because of embolic and mycotic complications, surgical intervention for myomectomy and removal of vegetations to prevent recurrent episodes of IE and systemic embolic effect was indicated. This case is not only a rare pediatric HOCM with IE, but it also demonstrates the invaluable role of echocardiography in the diagnosis of HOCM and its complications, especially in the case of IE. REFERENCES 1. Mazzoli M, Tefani 0, Vergassola R: Infective endocarditis: A complication of idiopathic hypertrophic subaortic stenosis (author’s translation). G Ital Cardiol 10:1228- 1233, 1980. 2. Novikov IuI, Polubentseva EI, Bespalova GN: Infectious endocarditis in hypertrophic cardiomyopathy. Ter Arkh 62235-89, 1990. 3. Stulz P, Zimmerli W, Mihatsch J , et al: Recurrent infective endocarditis in idiopathic hypertrophic subaortic stenosis. Thorac Cardiouasc Surg 37: 99- 102, 1989. 4. Carpenter PM, Atai M, Hoit B: Aneurysm of the mitral valve in a patient with hypertrophic cardiomyopathy. A m J Cardiouasc Pathol 2:273-276, 1988. 5. Winkelmann M, Curtius JM, Hoffmann V, et al:
Infectious endocarditis in hypertrophic obstructive cardiomyopathy. Z Kardiol 75:505-508, 1986. 6. Khekimian A, Pavlov TS: Case of infectious endocarditis in hypertrophic cardiomyopathy. Vutr Boles 25:104-107, 1986. 7. Petitalot JP, Allal J , Bordage J P , et al: Diagnosis and echocardiographic course of infectious endocarditis in obstructive cardiomyopathy. Ann Cardiol Angeiol (Paris) 34:353-356, 1985. 8. Zimlichman R, Ovsyshcher IA: Infective endocarditis in hypertrophic cardiomyopathy: Case report and review of literature 1961-1982. Mt Sinai J Med 51:614-619, 1984. 9. Ovsyshcher IA, Zimlichman R: Infective endocarditis in hypertrophic cardiomyopathy secondary to amiodarone treatment [letter]. Chest 832333, 1983. 10. Martinez-Orozco F, Ancochea L, Valls V, et al: Subacute infectious endocarditis in a n elderly patient suffering from obstructive cardiomyopathy. Rev Clin Esp 166:245-247, 1982. 11. Moiseev VS, Zhdanova NS, Kosmachev NN, et al: Infectious endocarditis in hypertrophic (muscular) subaortic stenosis. Klin Med (Mosk) 60238-91, 1982. 12. Chagnac A, Rudniki C, Loebel H, et al: Infectious endocarditis in idiopathic hypertrophic subaortic stenosis: Report of three cases and review of the literature. Chest 81:346-349, 1982. 13. McKinsey DS, Ratts TE, Bisno AL: Underlying cardiac lesions in adults with infective endocarditis. The changing spectrum. Am J Med 82:681688, 1987. 14. Lutas EM, Roberts RB, Devereaux RB, Prieto LM: Relation between the presence of echocardiographic vegetations and the complication rate in infective endocarditis. Am Heart J 112:107-113, 1986.
JOURNAL
OF CLINICAL ULTRASOUND
